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Cystic Fibrosis - Europe PMC

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Last Updated: 23 June 2022

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Living with cystic fibrosis during the COVID-19 pandemic: a social connectedness perspective.

Social connectedness is an essential component of belonging, and it refers to an individual's sense of belonging in the social world. Participants demonstrated resilience in the face of adversity, highlighted empathy in connection with the pandemic, and discussed how social involvement is both an outcome and a source of social network. Conclusions This book contributes to scant social connectivity literature on CF and chronic illness in general, highlighting the importance of increasing, assessing, and interventions in CF healthcare both within and outside the COVID-19 pandemic.

Source link: https://europepmc.org/article/MED/35438049


Pseudomonas aeruginosa adaptation in cystic fibrosis patients increases C5a levels and promotes neutrophil recruitment.

The symptoms of Cystic fibrosis disease are characterized by a strong airway inflammation response mediated by neutrophils and persistent respiratory infections caused by P. aeruginosa. We investigated how the isolates from CF patients' C5a levels can be modified and established the bacterial causes involved in this research. These results show that the non-cleaving C5a phenotype found in CF patients is not causal, contributing to increased inflammation and reduced bacterial clearance in CF patients.

Source link: https://europepmc.org/article/MED/35094639


Strain-specific predation of Bdellovibrio bacteriovorus on Pseudomonas aeruginosa with a higher range for cystic fibrosis than for bacteremia isolates.

This study was designed to determine the predatory activity of Bdellovibrio bacteriovorus 109J on clinical isolates of Pseudomonas aeruginosa and bloodstream infections, including strains isolated by genetic lineage, antibiotic resistance phenotype, and colony phenotype. In comparison to the initial inoculum, the predation yield is the ratio of viable prey cells remaining after predation. Specifically for CF strains was higher than for BSI P. aeruginosa isolates, presumably due to an environmental cause of CF strains, although invasive strains are more adapted to humans. A polyhydroxyloate depolymerase deficient B. bacteriovorus bd2637 mutant was used to determine the likelihood of increasing I-PR of BSI isolates.

Source link: https://europepmc.org/article/MED/35732651


Emerging medicines to improve the basic defect in cystic fibrosis.

CFTR modulators aimed at restoring the CFTR protein function have been available over the past ten years, and they will benefit about 80% of patients with CF. However, more than 5% of CFTR mutations do not have a CFTR protein for CFTR modulators to act on. The design of CFTR modulators and their use in patients with CF will be investigated. Then, the various approaches to treat patients with mutations that are non-responsive to CFTR modulators will be discussed. Since the introduction of CFTR modulators, CF disease has drastically changed.

Source link: https://europepmc.org/article/MED/35731915


Exercise versus airway clearance techniques for people with cystic fibrosis.

Background Tones There are numerous approved airway clearance methods for treating the respiratory health of people with cystic fibrosis, but none of which demonstrate superiority. Also, one of the top ten research questions raised by people with CF is whether exercise will substitute other ACTs. A safe and efficient ACT will help people with CF decide whether to incorporate this strategy into their treatment plans and possibly reduce their treatment burdens. The timing of this review is particularly important given the changing landscape of CF management with the introduction of highly-effective small molecule therapies, which are changing the way people with CF are cared for. Objects: To compare the effect of exercise to other ACTs for improving respiratory function and other medical outcomes in people with CF, as well as evaluating potential adverse effects of this ACT. For at least two therapy sessions in people with CF, there were randomised controlled trials and quasi-RCTs comparing exercise to another ACT. Exercise alone to ACBT can be used in a cross-over study. We saw no change from baseline in our primary outcome FEV 1; however, it increased in the endurance group before returning to baseline after 30 minutes; we are uncertain if exercise affected FEV 1 because the evidence is very poor; we are uncertain if exercise affected FEV 1. Exercise plus postural drainage and percussion versus PD&P only Two studies compared exercise and PD&P to PD&P alone, with augmentation and percussion.

Source link: https://europepmc.org/article/MED/35731672


Tissue-localized immune responses in people with cystic fibrosis and respiratory nontuberculous mycobacteria infection.

Nontuberculous mycobacteria are a common cause of respiratory disease in people with cystic fibrosis. Many with no history of NTM infection, PwCF, or a history of NTM infection are more likely to suffer severe respiratory disease and have difficulty regarding treatment. These findings' implications for our knowledge of lung disease in PwCF are discussed, as well as how they can influence the development of host-directed therapies to improve NTM disease treatment.

Source link: https://europepmc.org/article/MED/35536650


Beneficial short-term effect of autogenic drainage on peripheral resistance in childhood cystic fibrosis disease.

Background Airway clearance methods are supposed to be a vital component in the improvement of cystic fibrosis's improved peripheral clearance. Methods Thirty children with CF in a stable condition underwent IOS testing at baseline and after AD. With the Wilcoxon test, a Paired-comparison of AD's effects on airway resistance measurements was performed. During the session, the congestion scale related to the wet weight of sputum production. The eRIC model enabled the estimation of the respiratory system's peripheral resistance, which also decreased in the children with congestion, but not improved in the children without congestion. Conclusion One session of autogenic drainage has the ability to reduce peripheral resistance obtained from IOS results, more specifically in children with moderate to severe bronchial congestion.

Source link: https://europepmc.org/article/MED/35729620


Synergistic Activity of Repurposed Peptide Drug Glatiramer Acetate with Tobramycin against Cystic Fibrosis Pseudomonas aeruginosa.

Pseudomonas aeruginosa is the most common pathogen affecting lungs of people with cystic fibrosis causing acute and chronic infections. P. aeruginosa can colonize and persist in spite of antibiotic therapy, thanks to intrinsic and acquired antibiotic resistance, as well as physical barriers resulting from desiccated CF sputum. P. aeruginosa is also vulnerable to the wider global issue of antimicrobial resistance as well as the specific difficulties in eradicating P. aeruginosa from CF lungs. GA improved the efficiency of tobramycin against reference strains of P. aeruginosa, lowering TOB MICs and synergizing with the aminoglycoside, according to a growth, viability, MIC findings, and synergy study. This was also the case with clinical strains from people with CF. Compared to TOB-only studies, GA dramatically reduced the MIC 50 of viable cells from 1. 69 mg/L to 2. 20 mg/L, leading to MIC 90 results. GA resulted in significant changes in outer membranes, depolarization of cytoplasmic membranes, and permeabilization of P. aeruginosa, according to the only agent tested to significantly influence all three mechanisms. glatiramer acetate, a peptide drug that functions like many antimicrobial peptides, interfering with and damaging the P. aeruginosa cell wall and permeabilizing bacterial cells, allowing tobramycin to work as a peptide drug. Our results show that glatiramer acetate, as an antibiotic resistance breaker for pathogenic P. aeruginosa, is a good candidate for repurposing.

Source link: https://europepmc.org/article/MED/35727066


Macrophage PD-1 associates with neutrophilia and reduced bacterial killing in early cystic fibrosis airway disease.

Macrophages are the key resident immune cells in human airways that control responses to disease and injury coordinated responses. We therefore sought to find potential connections between macrophage PD-1 and airway disease markers in children with CF. By computed tomography, physical and bronchoalveolar lavage fluid from 45 children with CF from 3 to 62 months was determined, and structural lung damage was quantified by computed tomography. In an in vitro model mimicking leukocyte recruitment into CF airways, soluble mediators derived from recruited neutrophils induced PD-1 expression on recruited monocytes/macrophages, establishing a causal correlation between neutrophilic inflammation and macrophage PD-1 expression in CF. Eventually, blockade of PD-1 in a short-term culture of CF BALF leukocytes resulted in improved pathogen clearance. These findings, taken together, show that early CF lung disease, PD-1 upregulation partners are affected by airway macrophage exhaustion, neutrophil takeover, infection, and structural harm in early CF lung disease, indicating an early CF lung disease, PD-1 upregulation associates with airway macrophage exhaustion, neutrophil mistrophy, inflammation, and structural harm.

Source link: https://europepmc.org/article/MED/35732550

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions